Stanley Charles CV 1 2015_Redactedh

UNIVERSITY OF PENNSYLVANIA - PERELMAN SCHOOL OF MEDICINE

Curriculum Vitae

Date: 01/05/2015

Charles A. Stanley, M.D.

Address: Children's Hospital of Philadelphia

34th Street & Civic Center Blvd

Philadelphia, PA 19104 USA

Education:

1964 B.A. Harvard University (Biochemical Sciences)

1970 M.D. University of Virginia School of Medicine (Medicine)

Postgraduate Training and Fellowship Appointments:

1970-1972 Resident in Pediatrics, Children's Hospital of Philadelphia

1972-1975 Fellowship in Metabolic Diseases, Children's Hospital of

Philadelphia

Faculty Appointments:

1972-1976 Instructor in Pediatrics, Department of Pediatrics, University of

Pennsylvania School of Medicine

1976-1982 Assistant Professor of Pediatrics, University of Pennsylvania

School of Medicine

1982-1989 Associate Professor of Pediatrics at the Children's Hospital of

Philadelphia, University of Pennsylvania School of Medicine

1989-2011 Professor of Pediatrics at the Children's Hospital of

Philadelphia, University of Pennsylvania School of Medicine

2011-present Emeritus Professor CE of Pediatrics, University of

Pennsylvania School of Medicine

Hospital and/or Administrative Appointments:

1975-1982 Associate Physician, Endocrine/Diabetes Division, Children's

Hospital of Philadelphia

1982-present Senior Physician, Endocrine/Diabetes, Children's Hospital of

Philadelphia

1982-1994 Associate Director, General Clinical Research Center,

Children's Hospital of Philadelphia

1994-2012 Core Laboratory Director, Clinical Translational Research

Center, Children's Hospital of Philadelphia

(b) (6)

(b) (6)

Charles A. Stanley, M.D. Page 2

2000-2008 Chief, Division of Endocrinology/Diabetes, Children's Hospital

of Philadelphia

Other Appointments:

[none]

Specialty Certification:

1976 American Board of Pediatrics (19045)

1978 Pediatric Endocrinology (200)

Licensure:

1970-present Pennsylvania (MD-013278-E)

Awards, Honors and Membership in Honorary Societies:

[none]

Memberships in Professional and Scientific Societies and Other Professional Activities:

National:

1976-Present Lawson Wilkins Pediatric Endocrine Society (Drug and Therapeutics Committee, 1997

- 2000; Board of Directors, 2005 - 2009; President, 2008 - 2009; Chair of the Awards

Committee, 2009)

1987-Present American Diabetes Association (Philadelphia Affiliate, President of Professional

Section (1987-1988); member of Executive Committee)

2009-Present Congenital Hyperinsulinism International (Scientific Advisory Board)

American Association for the Advancement of Science

American Pediatric Society

Association for Glycogen Storage Disease

Endocrine Society

John Morgan Society, University of Pennsylvania

Society for Inherited Metabolic Disorders

Society for Pediatric Research

Editorial Positions:

1981-Present NICHD Site Visitor, Cleveland, Ohio

1983-Present NIH Site Visitor, GCRC, Duke University

1985-Present NIH Site Visitor, GCRC, Cleveland


1986-Present NIH Site Visitor, GCRC, Cincinnati

1994-Present NIH Medical Biochemistry Study Section, Ad Hoc Reviewer

2000-2008 Associate Editor, Diabetes

2004-2010 Editorial Board, Journal of Pediatrics

2013-Present FDA Endocrinology Metabolism Diabetes Advisory Committee

Academic and Institutional Committees:

1985-present GCRC Advisory Committee (Ex officio)

1989-1997 CHOP Internal Review Board

2000-Present DERC Executive Committee

Major Academic and Clinical Teaching Responsibilities:

[none]

Lectures by Invitation (Last 5 years):

Feb, 2010 "Genetic Disorders of Insulin Secretion in Infants: Over and Under",

Grand Rounds Baylor College of Medicine, Houston, TX

Mar, 2010 "Genetic Forms of Hyperinsulinemic Hypoglycemia in Neonates and

Children", Pediatric Grand Rounds, UMDNJ

May, 2010 "Congenital Hyperinsulinism Hypoglycemia: from bedside to bench",

Capital Medical University, Beijing Children's Hospital, Medical

Continued Educational Course and Pediatric Endo Forum, Beijing,

China

May, 2010 "GDH dysregulation in genetic forms of hyperinsulinemic

hypoglycemia" Symposium on Glutamate Dehydrogenase and

Human Disease and the Sixth Aegean Meeting on Neurological

Therapeutics, Heraklion, Crete, Greece

Jan, 2011 "Hypoglycemia", Neonatology Fellowship Physiology Lecture, The

Children's Hospital of Philadelphia

Feb, 2011 "Diagnosis and management of neonatal hypoglycemia(s)", New

York University Pediatric Grand Rounds, New York

Feb, 2012 "Management of Glucose in the Preterm Infant",NEO Conference,

Orlando, FL

Mar, 2012 "Welcome, Introductions and Symposium Overview",

KATP-channel Hyperinsulinism Variations", Glucokinase

Hyperinsulinism", "Perinatal Stress Hyperinsulinism", Monogenic

Disorder of Insulin Secretion: Congenital Hyperinsulinism and

Neonatal Diabetes Symposium, The Children's Hospital of

Philadelphia

Apr, 2012 "Diagnosis and Management of Hypoglycemia in Infants and

Children", George H. Lowrey Lecture in Pediatric Endocrinology

Grand Rounds, University of Michigan, Ann Arbor, MI

Mar, 2013 "Monogenic Disorders of Hyperinsulinemic Hypoglycemia", Grand

Rounds, University of Chicago, Chicago, IL

May, 2013 "Hypoglycemia in High Risk Neonates" Neonatal Nursing

Symposium, The Children's Hospital of Philadelphia


Nov, 2013 "Monogenic Disorders of Insulin Secretion: Congenital

Hyperinsulinism", NIH NICHD Grand Rounds, Bethesda, MD

Jan, 2014 "Hypoglycemia", Neonatal-Perinatal Fellows Physiology Course,

The Children's Hospital of Philadelphia

Apr, 2014 "Diagnosis and management of hypoglycemia in neonates, infants,

and children", Grand Rounds at Children's Hospital of Pittsburgh of

UPMC

"Monogenic disorders of hyperinsulinemic hypoglycemia in children

(and adults!)", Joint Adult-Pediatric Endocrinology Seminar, UPCM

"Strategies for management and discovery of congenital

hyperinsulinism disorders", Ped Endo Conference, Children's

Hospital of Pittsburgh

Aug, 2014 "Genetics and the History of Congenital Hyperinsulinism",

Congential Hyperinsulinism International Family Conference,

Gallaoway, NJ

Dec, 2014 The Hypoketotic Hypoglycemias: "(Holmes..the curious incident of

the dog in the night-time..." Pediatric Grand Rounds, The Children's

Hospital of Philadelphia

Jan, 2015 "Diagnosis and Management of Hypoglycemia in Neonates, Infants,

and Children" All Children's Hospital Grand Rounds, Johns Hopkins

Medicine, St. Petersburg, Florida

Organizing Roles in Scientific Meetings:

Jun, 2006 Symposium Director - Congenital Hyperinsulinism and Related

Disorders of Insulin Secretion: Clinical, Biochemical and Genetic

Advances.

Philadelphia, PA

Mar, 2012 Symposium Course Director - Monogenic Disorders of Insulin

Secretion: Congenital Hyperinsulinism and Neonatal Diabetes

The Children's Hospital of Philadelphia

Bibliography:

Research Publications, peer reviewed (print or other media):

1. Chun PW, Kim SJ, Stanley CA, Ackers G: Determination of the equilibrium constants of

associating protein systems III Evaluation of the weight fraction of monomer from

the weight-average partition coefficient (application to bovine liver glutamate

dehydrogenase). Biochem 8: 1625-1632, 1969.

2. Stanley CA, Baker L: Accidental poisoning with 50 percent glucose solution: The

danger of large stock bottles. J Pediatr 84: 270-71, 1974.

3. Ranke MB, Stanley CA, Robard D, Baker L, Bongiovanni A, Parks JS: Sex differences

in binding of human growth hormone to isolated rat hepatocytes. Proc Natl Acad

Sci USA 73: 847-51, 1976.


4. Ranke MB, Stanley CA, Tenore A, Robard D, Bongiovanni AM, Parks JS:

Characterization of somatogenic and lactogenic binding sites in isolated rat

hepatocytes. Endocriniol 99: 1033-1045, 1976.

5. Stanley CA, Baker L: Hyperinsulinism in infancy: Diagnosis by demonstration of

abmornal response to fasting hypoglycemia. Pediatrics 57: 702-11, 1976.

6. Gauderer M, Stanley CA, Baker L, Bishop HC: Pancreatic adenomas in infants and

children: Current surgical management. J Pediatr Surg 13: 591-96, 1978.

7. Gardner DF, Kaplan MM, Stanley CA, Utiger RD: Effect of tri-iodothyronine

replacement on the metabolic and pituitary responses to starvation. N Engl J Med

300: 579-584, 1979.

8. Gardner DF, Sterling FH, Stanley CA: Pancreatic venography and plasma ketone

measurements in the diagnosis of hyperinsulinism. Am J Med Sci 277: 23-27,

1979.

9. Stanley CA, Anday EK, Baker L, Delivoria-Papadopoulos M : Metabolic fuel and

hormone reposes to fasting in newborn infants. Pediatrics 64: 613-19, 1979.

10. Stanley CA, Spielman RS, Zmijewski CM, Baker L: Wolfram syndrome not HLA

linked. N Engl J Med 301: 1389, 1979 Notes: letter to the editor.

11. Finegold DN, Stanley CA, Baker L: Glycemic response to glucagon during fasting

hypoglycemia: An aid in the diagnosis of hyperinsulinism. J Pediatr 96: 257-59,

1980.

12. Pereira GR, Fox WW, Stanley CA, Baker L, Schwartz MS: Decreased oxygenation and

hyperlipemia during intravenous fat infusions in premature infants. Pediatrics 66:

26-30, 1980.

13. Anday EK, Stanley CA, Baker L, Delivoria-Papadopoulos M: Plasma ketones in

newborn infants: Absence of suckling ketosis. J Pediatr 98: 628-30, 1981.

14. Slonim AE, Borum PR, Tanaka K, Stanley CA, Kasselberg AG, Greene HL, Burr IM:

Dietary-dependent carnitine deficiency as a cause of nonketotic hypoglycemia in

an infant. J Pediatr 99: 551-56, 1981.

15. Stanley CA, Mills JL, Baker L: Intragastric feeding in Type I glycogen storage disease:

Factors affecting the control of lactic acidemia. Pediatr Res 15: 1504-08, 1981.

16. Kenepp NB, Kumar S, Shelley WC, Stanley CA, Gabbe SG, Gutsche BB: Fetal and

neonatal hazards of maternal hydration with 5% dextrose before cesarean section.

Lancet 1: 1150-52, 1982.


17. Kennedy TL, Stanley CA, Baker L, Norman ME: Propranolol-induced hypoglycemia

and hypertension in a child. Internat J Pediatr Nephrol 2: 255, 1982.

18. Stanley CA, Gonzales E, Baker L: Development of hepatic fatty acid oxidation and

ketogenesis in the newborn guinea pig. Pediatr Res 17: 224-29, 1983.

19. Stanley CA, Hale DE, Coates PM, Hall CL, Corkey BE, Yang W, Kelley RI, Gonzales

BS, Williamson JR, Baker L: Medium-chain acyl-CoA dehydrogenase deficiency

in children with non-ketotic hypoglycemia and low carnitine levels. Pediatr Res

17: 877-84, 1983.

20. Wright LL, Stanley CA, Anday EK, Baker L: The effect of early feeding on plasma

glucose levels in SGA infants. Clin Pediatr 22: 539-41, 1983.

21. Yudkoff M, Nissim I, Stanley CA, Baker L, Segal S: Glycogen storage disease: Effects

of glucose infusions on (15N) glycine kinetics and nitrogen metabolism. J Pediatr

Gastroenterol Nutr 3: 81-88, 1984.

22. Coates PM, Hale DE, Stanley CA, Corkey BE, Cortner JA: Genetic deficiency of

medium-chain acyl coenzyme a dehydrogenase. Studies in cultured skin

fibroblasts and peripheral mononuclear leukocytes. Pediatr Res 19: 671-76, 1985.

23. Hale DE, Batshaw ML, Coates PM, Frerman FE, Goodman SI, Singh I, Stanley CA:

Long-chain acyl CoA dehydrogenase deficiency: an inherited cause of non-ketotic

hypoglycemia. Pediatr Res 19: 666-71, 1985.

24. Fromm M, Littman P, Raney RB, Nelson L, Handler S, Diamond G, Stanley CA: Late

effects after Treatment of twenty children with soft tissue sarcomas of the head and

neck. Cancer 57: 2070-76, 1986.

25. Koven NL, Clark MM, Cody CS, Stanley CA, Baker L, Douglas SD: Impaired

chemotaxis and neutrophil (polymorphonuclear leukocyte) function in

glycogenesis type IB. Pediatr Res 20: 438-42, 1986.

26. The DCCT Research Group: The diabetes control and complications trial (DCCT):

Design and methodologic considerations for the feasibility phase. Diabetes 35:

530-45, 1986.

27. Treem WR, Witzleben CA, Piccoli DA, Stanley CA, Hale DE, Coates PM, Watkins JB:

Medium-chain and long-chain acyl CoA dehydrogenase deficiency: Clinical,

pathologic, and ultrastructural differentiation from Reye's syndrome. Hepatology

6: 1270-78, 1986.

28. Van Dyke DC, Quinn GE, Bruce DA, Stanley CA: Clinical management

considerations in uncommon midline central nervous system anomalies. Dysmorph

Clin Genet 1: 6-11, 1987.


29. Corkey BE, Hale DE, Glennon MC, Kelley RI, Coates PM, Kilpatrick L, Stanley CA:

Relationship between unusual hepatic acyl coenzyme A profiles and the

pathogenesis of Reye's syndrome. J Clin Invest 82: 782-788, 1988.

30. Rinaldo P, O'Shea JJ, Coates PM, Hale DE, Stanley CA, Tanaka K: Medium chain

acyl-CoA dehydrogenase deficiency: Diagnosis by stable-isotope dilution

measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine. N Engl

J Med 319: 1308-13, 1988.

31. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine

deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts.

N Engl J Med 319: 1331-36, 1988.

32. Baker L, Dahlem S, Goldfarb S, Kern EF, Stanley CA., Egler J, Olshan JS, Heyman S:

Hyperfiltration and renal disease in glycogen storage disease, type I. Kidney Int 35:

1345-50, 1989.

33. Belik J, Wagerle LC, Stanley CA, Sacks LM, Hergert DW, Delivoria-Papadopoulis M:

Cerebral metabolic response and mitochondrial activity following insulin-induced

hypoglycemia in newborn lambs. Biol Neonate 55: 281-89, 1989.

34. Bergman I, Finegold D, Gartner JC, Zitelli BJ, Classen D, Scarano J, Roe CR, Stanley

CA, Goodman SI: Acute profound dystonia in infants with glutaric acidemia.

Pediatrics 83: 228-33, 1989.

35. Treem WR, Stanley CA: Massive hepatomegaly, steatosis, and secondary plasma

carnitine deficiency in an infant with cystic fibrosis. Pediatrics 83: 993-97, 1989.

36. Treem WR, Stanley CA, Goodman SI: Medium chain acyl-CoA dehydrogenase

deficiency: Metabolic effects and therapeutic efficacy of long-term L-carnitine

supplementation. J Inher Metab Dis 12: 112-19, 1989.

37. Kilpatrick L, Garty B-Z, Lundquist KF, Stanley CA, Baker L, Douglas SD, Korchak

HM: Impaired metabolic function and signaling defects in phagocytic cells in

glycogen storage disease type 1B. J Clin Invest 86: 196-202, 1990.

38. Baker L, Thornton PS, Stanley CA: Management of hyperinsulinism in infants. J

Pediatr 119: 755-758, 1991 Notes: editorial.

39. Bennett MJ, Hale DE, Coates PM, Stanley CA: Postmortem recognition of fatty acid

oxidation disorders. Peditr Path 11: 365-70, 1991.


40. Stanley CA, DeLeeuw S, Coates PM, Vianey-Liaud C, Divry P, Bonnefont J-P,

Saudubray J-M, Haymond M, Trefz FK, Breningstall GN, Wappner RS, Byrd DJ,

Sansaricq C, Tein I, Grover W, Valle D, Rutledge SL, Treem WR: Chronic

cardiomyopathy and weakness or acute coma in children with a defect in carnitine

uptake. Ann Neurol 30: 709-16, 1991.

41. Thornton PS, Summer AE, Ruchelli ED, Spielman RS, Baker L, Stanley CA: Familial

and sporadic hyperinsulinism: Histopathology and segregation analysis support a

single autosomal recessive disorder. J Pediatr 119: 721-24, 1991.

42. Treem WR, Stanley CA, Hale DE, Leopold HB, Hyams JS: Hypoglycemia, hypotonia,

and cardiomyopathy: The evolving clinical picture of long-chain acyl-CoA

dehydrogenase deficiency. Pediatrics 87: 328-33, 1991.

43. Stanley CA, Hale DE, Berry GT, Deleeuw S, Boxer J, Bonnefont JP: Brief report: a

deficiency of carnitine-acylcarnitine translocase in the inner mitochondrial

membrane. N Engl J Med 327(1): 19-23, Jul 1992.

44. Schmidt-Sommerfeld E, Penn D, Duran M, Rinaldo P, Bennett MJ, Santer R, Stanley

CA: Detection and quantitation of acylcarnitines in plasma and blood spots from

patients with inborn errors of fatty acid oxidation. Prog Clin Biol Res 375: 355-62,

1992.

45. Stanley CA, Sunaryo F, Hale DE, Bonnefont JP, Demaugre F, Saudubray JM: Elevated

plasma carnitine in the hepatic form of carnitine palmitoyltransferase-1 deficiency.

J Inherit Metab Dis 15(5): 785-9, 1992.

46. Restaino I, Kaplan BS, Stanley C, Baker L: Nephrolithiasis, hypocitraturia, and a distal

renal tubular acidification defect in type 1 glycogen storage disease. J Pediatr

122(3): 392-6, Mar 1993.

47. Schmidt-Sommerfeld E, Penn D, Duran M, Bennett MJ, Santer R, Stanley CA:

Detection of inborn errors of fatty acid oxidation from acylcarnitine analysis of

plasma and blood spots with the radioisotopic exchange-high-performance liquid

chromatographic method. J Pediatr 122(5 Pt 1): 708-14, May 1993.

48. Stanley CA, Berry GT, Bennett MJ, Willi SM, Treem WR, Hale DE: Renal handling of

carnitine in secondary carnitine deficiency disorders. Pediatr Res 34(1): 89-97, Jul

1993.

49. McCawley LJ, Korchak HM, Cutilli JR, Stanley CA, Baker L, Douglas SD, Kilpatrick

L: Interferon-gamma corrects the respiratory burst defect in vitro in

monocyte-derived macrophages from glycogen storage disease type 1b patients.

Pediatr Res 34(3): 265-9, Sep 1993.


50. Thornton PS, Alter CA, Katz LE, Baker L, Stanley CA: Short- and long-term use of

octreotide in the treatment of congenital hyperinsulinism. J Pediatr 123(4): 637-43,

Oct 1993.

51. Korchak HM, Garty BZ, Stanley CA, Baker L, Douglas SD, Kilpatrick L: Impairment

of calcium mobilization in phagocytic cells in glycogen storage disease type 1b.

Eur J Pediatr 152 Suppl : S39-43, 1993.

52. McCawley LJ, Korchak HM, Douglas SD, Campbell DE, Thornton PS, Stanley CA,

Baker L, Kilpatrick L: In vitro and in vivo effects of granulocyte

colony-stimulating factor on neutrophils in glycogen storage disease type 1B:

granulocyte colony-stimulating factor therapy corrects the neutropenia and the

defects in respiratory burst activity and Ca2+ mobilization. Pediatr Res 35(1):

84-90, Jan 1994.

53. Treem WR, Rinaldo P, Hale DE, Stanley CA, Millington DS, Hyams JS, Jackson S,

Turnbull DM: Acute fatty liver of pregnancy and long-chain

3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Hepatology 19(2): 339-45,

Feb 1994.

54. Glaser B, Chiu KC, Anker R, Nestorowicz A, Landau H, Ben-Bassat H, Shlomai Z,

Kaiser N, Thornton PS, Stanley CA, Spielman RS, Gogolin-Ewens K, Cerasi E,

Baker L, Rice J, Donis-Keller H, Permutt MA: Familial hyperinsulinism maps to

chromosome 11p14-15.1, 30 cM centromeric to the insulin gene. Nat Genet 7(2):

185-8, Jun 1994.

55. Baker L, Stanley CA: Neonatal hypoglycemia. Curr Ther Endocrinol Metab 5:

376-80, 1994.

56. Stayer SA, Steven JM, Nicolson SC, Jobes DR, Stanley CA, Baumgart S: The

metabolic effects of surface cooling neonates prior to cardiac surgery. Anesth

Analg 79: 834-39, 1994.

57. Glaser B, Chiu KC, Liu L, Anker R, Nestorowicz A, Cox NJ, Landau H, Kaiser N,

Thornton PS, Stanley CA, Cerasi E, Baker L, Donis-Keller H, Permutt MA:

Recombinant mapping of the familial hyperinsulinism gene to an 0.8 cM region on

chromosome 11p15.1 and demonstration of a founder effect in Ashkenazi Jews.

Hum Mol Genet 4(5): 879-86, May 1995.

58. Rutledge SL, Berry GT, Stanley CA, van Hove JL, Millington D: Glycine and

L-carnitine therapy in 3-methylcrotonyl-CoA carboxylase deficiency. J Inherit

Metab Dis 18(3): 299-305, 1995.

59. Bennett MJ, Hale DE, Pollitt RJ, Stanley CA, Variend S: Endocardial fibroelastosis

and primary carnitine deficiency due to a defect in the plasma membrane carnitine

transporter. Clin Cardiol 19(3): 243-6, Mar 1996.


60. Nestorowicz A, Wilson BA, Schoor KP, Inoue H, Glaser B, Landau H, Stanley CA,

Thornton PS, Clement JP, Bryan J, Aguilar-Bryan L, Permutt MA: Mutations in the

sulonylurea receptor gene are associated with familial hyperinsulinism in

Ashkenazi Jews. Hum Mol Genet 5(11): 1813-22, Nov 1996.

61. Treem WR, Shoup ME, Hale DE, Bennett MJ, Rinaldo P, Millington DS, Stanley CA,

Riely CA, Hyams JS: Acute fatty liver of pregnancy, hemolysis, elevated liver

enzymes, and low platelets syndrome, and long chain 3-hydroxyacyl-coenzyme A

dehydrogenase deficiency. Am J Gastroenterol 91(11): 2293-300, Nov 1996.

62. Christodoulou J, Teo SH, Hammond J, Sim KG, Hsu BY, Stanley CA, Watson B, Lau

KC, Wilcken B: First prenatal diagnosis of the carnitine transporter defect. Am J

Med Genet 66(1): 21-4, Dec 1996.

63. Lieu YK, Hsu BY, Price WA, Corkey BE, Stanley CA: Carnitine effects on coenzyme

A profiles in rat liver with hypoglycin inhibition of multiple dehydrogenases. Am J

Physiol 272(3 Pt 1): E359-66, March 1997.

64. Weinzimer SA, Stanley CA, Berry GT, Yudkoff M, Tuchman M, Thornton PS: A

syndrome of congenital hyperinsulinism and hyperammonemia. J Pediatr 130(4):

661-4, Apr 1997.

65. Chalmers RA, Stanley CA, English N, Wigglesworth JS: Mitochondrial

carnitine-acylcarnitine translocase deficiency presenting as sudden neonatal death.

J Pediatr 131(2): 220-25, August 1997.

66. Levitt Katz LE, Satin-Smith MS, Collett-Solberg P, Thornton PS, Baker L, Stanley

CA, Cohen P: Insulin-like growth factor binding protein-1 levels in the diagnosis of

hypoglycemia caused by hyperinsulinism. J Pediatr 131(2): 193-7, August 1997.

67. Rinaldo P, Stanley CA, Hsu BY, Sanchez LA, Stern HJ: Sudden neonatal death in

carnitine transporter deficiency. J Pediatr 131(2): 304-5, Aug 1997.

68. Thompson GN, Hsu BY, Pitt JJ, Treacy E, Stanley CA: Fasting hypoketotic coma in a

child with deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase.

N Engl J Med 337(17): 1203-7, Oct 1997.

69. Nestorowicz A, Inagaki N, Gonoi T, Schoor KP, Wilson BA, Glaser B, Landau H,

Stanley CA, Thornton PS, Seino S, Permutt MA: A nonsense mutation in the

inward rectifier potassium channel gene, Kir6.2, is associated with familial

hyperinsulinism. Diabetes 49(11): 1743-8, Nov 1997.


70. Cheung VG, Gregg JP, Gogolin-Ewens KJ, Bandong J, Stanley CA, Baker L, Higgins

MJ, Nowak NJ, Shows TB, Ewens WJ, Nelson SF, Spielman RS:

Linkage-disequilibrium mapping without genotyping. Nat Genet 18(3): 225-30,

1998.

71. Glaser B, Kesavan P, Heyman M, Davis E, Cuesta A, Buchs A, Stanley CA, Thornton

PS, Permutt MA, Matschinsky FM, Herold KC: Familial hyperinsulinism caused

by an activating glucokinase mutation. N Engl J Med 338(4): 226-30, 1998.

72. Glaser B, Thornton PS, Herold K, Stanley CA: Clinical and molecular heterogeneity of

familial hyperinsulinism. J Pediatr 133(6): 801-2, 1998.

73. Iacobazzi V, Naglieri MA, Stanley CA, Wanders RJ, Palmieri F: The structure and

organization of the human carnitine/acylcarnitine translocase (CACT1) gene2.

Biochem Biophys Res Commun 252(3): 770-4, 1998.

74. Katz LE, Satin-Smith MS, Collett-Solberg P, Baker L, Stanley CA, Cohen P: Dual

regulation of insulin-like growth factor binding protein-1 levels by insulin and

cortisol during fasting. J Clin Endocrinol Metab 83(12): 4426-30, 1998.

75. Nestorowicz A, Glaser B, Wilson BA, Shyng SL, Nichols CG, Stanley CA, Thornton

PS, Permutt MA: Genetic heterogeneity in familial hyperinsulinism. Hum Mol

Genet 7(7): 1119-28, 1998.

76. Stanley CA: Dissecting the spectrum of fatty acid oxidation disorders. J Pediatr 132(3

Pt 1): 384-86, 1998.

77. Stanley CA, Lieu YK, Hsu BY, Burlina AB, Greenberg CR, Hopwood NJ, Perlman K,

Rich BH, Zammarchi E, Poncz M: Hyperinsulinism and hyperammonemia in

infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J

Med 338(19): 1352-57, 1998.

78. Thornton PS, Satin-Smith MS, Herold K, Glaser B, Chiu KC, Nestorowicz A, Permutt

MA, Baker L, Stanley CA: Familial hyperinsulinism with apparent autosomal

dominant inheritance: clinical and genetic differences from the autosomal recessive

variant. J Pediatr 132(1): 9-14, 1998.

79. Weinzimer SA, Stanley CA: Reply. J Pediatr 133(6): 801, December 1998.

80. Glaser B, Furth J, Stanley CA, Baker L, Thornton PS, Landau H, Permutt MA:

Intragenic single nucleotide polymorphism haplotype analysis of SUR1 mutations

in familial hyperinsulinism. Hum Mutat 14(1): 23-9, 1999.

81. Glaser, B Ryan F, Donath M, Landau H, Stanley CA, Baker L, Barton DE, Thornton

PS: Hyperinsulinism caused by paternal-specific inheritance of a recessive

mutation in the sulfonylurea-receptor gene. Diabetes 48(8): 1652-57, 1999.


82. Katz LE, Ferry RJ, Jr., Stanley CA, Collett-Solberg PF, Baker L, Cohen P. :

Suppression of insulin oversecretion by subcutaneous recombinant human

insulin-like growth factor I in children with congenital hyperinsulinism due to

defective beta-cell sulfonylurea receptor. J Clin Endocrinol Metab 84(9):

3117-24, 1999.

83. Lovvorn HN III, Nance ML, Ferry RJ Jr, Stolte L, Baker L, O'Neill JA Jr, Schnaufer L,

Stanley CA, Adzick NS: Congenital hyperinsulinism and the surgeon: lessons

learned over 35 years. J Pediatr Surg 34(5): 786-92, 1999.

84. Stanley CA, Baker L: The causes of neonatal hypoglycemia. N Engl J Med 340(15):

1200-01, 1999.

85. Ferry RJ Jr, Kelly A, Grimberg A, Koo-McCoy S, Shapiro MJ, Fellows KE, Glaser B,

Aguilar-Bryan L, Stafford DE, Stanley CA: Calcium-stimulated insulin secretion

in diffuse and focal forms of congenital hyperinsulinism. J Pediatr 137(2):

239-46, 2000.

86. Pierpont ME, Breningstall GN, Stanley CA, Singh A. : Familial carnitine transporter

defect: A treatable cause of cardiomyopathy in children. Am Heart J 139(2 Pt 3):

S93-S106, 2000.

87. Stanley CA, Fang J, Kutyna K, Hsu BY, Ming JE, Glaser B, Poncz M: Molecular basis

and characterization of the hyperinsulinism/hyperammonemia syndrome:

predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase

gene. HI/HA Contributing Investigators. Diabetes 49(4): 667-73, 2000.

88. Bouchard L, Robert MF, Vinarov D, Stanley CA, Thompson GN, Morris A, Leonard

JV, Quant P, Hsu BY, Boneh A, Boukaftane Y, Ashmarina L, Wang S, Miziorko H,

Mitchell GA: Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase

deficiency: clinical course and description of causal mutations in two patients.

Pediatr Res 2001 49(3): 326-31, 2001.

89. De Lonlay P, Benelli C, Fouque F, Ganguly A, Aral B, Dionisi-Vici C, Touati G,

Heinrichs C, Rabier D, Kamoun P, Robert JJ, Stanley C, Saudubray JM:

Hyperinsulinism and hyperammonemia syndrome: report of twelve unrelated

patients. Pediatr Res 50(3): 353-57, 2001.

90. Grimberg A, Ferry RJ Jr, Kelly A, Koo-McCoy S, Polonsky K, Glaser B, Permutt MA,

Aguilar-Bryan L, Stafford D, Thornton PS, Baker L, Stanley CA: Dysregulation of

insulin secretion in children with congenital hyperinsulinism due to sulfonylurea

receptor mutations. Diabetes 50(2): 322-28, 2001.


91. Hsu BY, Iacobazzi V, Wang Z, Harvie H, Chalmers RA, Saudubray JM, Palmieri F,

Ganguly A, Stanley CA: Aberrant mRNA splicing associated with coding region

mutations in children with carnitine-acylcarnitine translocase deficiency. Mol

Genet Metab 74(1-2): 248-55, 2001.

92. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA:

Protein-sensitive and fasting hypoglycemia in children with the

hyperinsulinism/hyperammonemia syndrome. J Pediatr 138(3): 383-89, 2001.

93. Kelly A, Ng D, Ferry RJ Jr, Grimberg A, Koo-McCoy S, Thornton PS, Stanley CA:

Acute insulin responses to leucine in children with the

hyperinsulinism/hyperammonemia syndrome. J Clin Endocrinol Metab 86(8):

3724-28, 2001.

94. MacMullen C, Fang J, Hsu BY, Kelly A, de Lonlay-Debeney P, Saudubray JM,

Ganguly A, Smith TJ, Stanley CA: Hyperinsulinism/hyperammonemia syndrome

in children with regulatory mutations in the inhibitory guanosine

triphosphate-binding domain of glutamate dehydrogenase. J Clin Endocrinol

Metab 86(4): 1782-87, 2001.

95. Smith TJ, Peterson PE, Schmidt T, Fang J, Stanley CA: Structures of bovine glutamate

dehydrogenase complexes elucidate the mechanism of purine regulation. J Mol

Biol 307(2): 707-20, 2001.

96. Cederbaum SD, Koo-McCoy S, Tein I, Hsu BY, Ganguly A, Vilain E, Dipple K,

Cvitanovic-Sojat L, Stanley C: Carnitine membrane transporter deficiency: a

long-term follow up and OCTN2 mutation in the first documented case of primary

carnitine deficiency. Mol Genet Metab 77(3): 195-201, 2002.

97. Fang J, Hsu BY, MacMullen CM, Poncz M, Smith TJ, Stanley CA: Expression,

purification and characterization of human glutamate dehydrogenase (GDH)

allosteric regulatory mutations. Biochem J 363(Pt 1): 81-87, 2002.

98. Smith TJ, Schmidt T, Fang J, Wu J, Siuzdak G, Stanley CA: The structure of apo

human glutamate dehydrogenase details subunit communication and allostery. J

Mol Biol 318(3): 765-77, 2002.

99. Suchi M, Thornton PS, Adzick NS, MacMullen C, Ganguly A, Stanley CA, Ruchelli

ED: Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the

extent of pancreatectomy. Am J Surg Pathol 28(10): 1326-35, Jul-Aug 2003.

100. Banerjee S, Schmidt T, Fang J, Stanley CA, Smith TJ: Structural studies on ADP

activation of mammalian glutamate dehydrogenase and the evolution of regulation.

Biochemistry 42(12): 3446-56, 2003.


101. Li C, Najafi H, Daikhin Y, Nissim IB, Collins HW, Yudkoff M, Matschinsky FM,

Stanley CA: Regulation of leucine-stimulated insulin secretion and glutamine

metabolism in isolated rat islets. J Biol Chem 278(5): 2353-58, 2003.

102. Suchi M, MacMullen C, Thornton PS, Ganguly A, Stanley CA, Ruchelli ED:

Histopathology of congenital hyperinsulinism: retrospective study with genotype

correlations. Pediatr Dev Pathol 6(4): 322-33, 2003.

103. Thornton PS, MacMullen C, Ganguly A, Ruchelli E, Steinkrauss L, Crane A,

Aguilar-Bryan L, Stanley CA: Clinical and molecular characterization of a

dominant form of congenital hyperinsulinism caused by a mutation in the

high-affinity sulfonylurea receptor. Diabetes 52(9): 2403-10, 2003.

104. Venditti LN, Venditti CP, Berry GT, Kaplan PB, Kaye EM, Glick H, Stanley CA:

Newborn screening by tandem mass spectrometry for medium-chain Acyl-CoA

dehydrogenase deficiency: a cost-effectiveness analysis. Pediatrics 112(5):

1005-15, 2003.

105. Stanley CA, Thornton PS, Ganguly A, MacMullen C, Underwood P, Bhatia P,

Steinkrauss L, Wanner L, Kaye R, Ruchelli E, Suchi M, Adzick NS: Preoperative

evaluation of infants with focal or diffuse congenital hyperinsulinism by

intravenous acute insulin response tests and selective pancreatic arterial calcium

stimulation. J Clin Endocrinol Metab 89(1): 288-96, January 2004.

106. Giurgea I, Laborde K, Touati G, Bellanne-Chantelot C, Nassogne MC, Sempoux C,

Jaubert F, Khoa N, Chigot V, Rahier J, Brunelle F, Nihoul-Fekete C, Dunne MJ,

Stanley C, Saudubray JM, Robert JJ, de Lonlay P: Acute insulin responses to

calcium and tolbutamide do not differentiate focal from diffuse congenital

hyperinsulinism. J Clin Endocrinol Metab 89(2): 925-929, February 2004.

107. Adzick NS, Thornton PS, Stanley CA, Kaye RD, Ruchelli E: A multidisciplinary

approach to the focal form of congenital hyperinsulinism leads to successful

treatment by partial pancreatectomy. J Pediatr Surg 39(3): 270-275, March

2004.

108. Li C, Buettger C, Kwagh J, Matter A, Daikhin Y, Nissim IB, Collins HW, Yudkoff M,

Stanley CA, Matschinsky FM: A signaling role of glutamine in insulin secretion. J

Biol Chem 279(14): 13393-401, April 2004.

109. Magge SN, Shyng SL, MacMullen C, Steinkrauss L, Ganguly A, Katz LE, Stanley

CA: Familial leucine-sensitive hypoglycemia of infancy due to a dominant

mutation of the beta-cell sulfonylurea receptor. J Clin Endocrinol Metab 89(9):

4450-56, September 2004.


110. Cucchiaro G, Markowitz SD, Kaye R, Adzick NS, Litman RS, Stanley CA, Watcha

MF: Blood glucose control during selective arterial stimulation and venous

sampling for localization of focal hyperinsulinism lesions in anesthetized children.

Anesth Analg 99(4): 1044-48, October 2004.

111. Suchi M, Thornton PS, Adzick NS, MacMullen C, Ganguly A, Stanley CA, Ruchelli

ED: Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the

extent of pancreatectomy. Am J Surg Pathol 28(10): 1326-35, Oct 2004.

112. Allen A, Kwagh J, Fang J, Stanley CA, Smith TJ. : Evolution of glutamate

dehydrogenase regulation of insulin homeostasis is an example of molecular

exaptation. Biochemistry 43(45): 14431-43, November 2004.

113. Henwood MJ, Kelly A, Macmullen C, Bhatia P, Ganguly A, Thornton PS, Stanley

CA: Genotype-Phenotype Correlations in Children with Congenital

Hyperinsulinism Due to Recessive Mutations of the KATP Channel Genes. J Clin

Endocrinol Metab November 2004.

114. Kelly A, Li C, Gao Z, Stanley CA, Matschinsky FM: Glutaminolysis and insulin

secretion: from bedside to bench and back. Diabetes 51(Suppl 3): S421-26, 2004.

115. Gloyn AL, Odili S, Zelent D, Castleden HA, Steele AM, Stride A, Magnuson MA,

Lorini R, d'Annunzio G, Stanley CA, Kwagh J, van Schaftingen E, Barbetti F, Han

Y, Grimsby J, Taub R, Ellard S, Hattersley AT, Matschinsky FM: Insights into the

structure and regulation of glucokinase from a novel mutation (V62M) which

causes maturity-onset diabetes of the young. J Bio Chem January 2005.

116. Raizen DM, Brooks-Kayal A, Steinkrauss L, Tennekoon GI, Stanley CA, Kelly A:

Central nervous system hyperexcitability associated with glutamate dehydrogenase

gain of function mutations. J Pediatr 146(3): 388-94, March 2005.

117. Steinkrauss L, Lipman TH, Hendell CD, Gerdes M, Thornton PA, Stanley CA:

Effects of hypoglycemia on developmental outcome in children with congenital

hyperinsulinism. Journal of Pediatric Nursing 20(2), April 2005.

118. Hoe FM, Thornton PS, Wanner LA, Steinkrauss L, Simmons RA, Stanley CA:

Clinical features and insulin regulation in infants with a syndrome of prolonged

neonatal hyperinsulinism. J Pediatr 148(2): 207-12, Feb 2006.

119. Li C, Allen A, Kwagh J, Doliba N, Qin W, Najafi H, Collins HW, Matschinsky FM,

Stanley CA, Smith TJ: Green tea polypenols modulate insulin secretion by

inhibiting glutamate dehydrogenase. Ameri Soc for Biochem and Molec Biol, Inc.

Feb 13 2006.


120. Lin YU, MacMullen C, Ganguly A, Stanley CA, Shyng SL: A novel KCNJ11

Mutation Associated with congenital hyperinsulinism reduces the intrinsic open

probability of beta-cell APT-sensitive potassium channels. J Biol Chem 281(5):

3006-12, Feb 2006.

121. Li C, Allen A, Kwagh J, Doliba N, qin W, Najafi H, Collins HW, Matschinsky FM,

Stanley CA, Smith TJ: Green tea polyphenols modulate insulin secretion by

inhibiting glutamate dehydrogenase. J Biol Chem 281: 10214-10221, April 2006.

122. Hussain K, Seppnen M, Nnt-Salonen K, Adzick NS, Stanley CA, Thornton P,

Minn H: The diagnosis of ectopic focal hyperinsulinism of infancy with

[18F]-Dopa Positron Emission Tomography. J Clin Endocrino Metabol 91(8):

2839-42, May 2006.

123. Li C, Matter A, Kelly A, Petty TJ, Najafi H, MacMullen C, Daikhin Y, Nissim I,

Lazarow A, Kwagh J, Collins HW, Hsu BYL, Nissim I, Yudkoff M, Matschinsky

FM, Stanley CA: Effects of a GTP-insensitive mutation of glutamate

dehydrogenase on insulin secretion in transgenic mice. J Biol Chem 281(22):

15064 - 72, June 2 2006.

124. Peranteau WH, Ganguly A, Steinmuller L, Thornton P, Johnson MP, Howel LJ,

Stanley CA, Adzick NS: Prenatal diagnosis and postnatal management of diffuse

congenital hyperinsulinism: a case report. Fetal Diagn Ther 21(6): 515-18, Sept 12

2006.

125. Fourtner SH, Stanley CA, Kelly A: Protein-sensitive hypoglycemia without leucine

sensitivity in hyperinsulinism caused by K(ATP) channel mutations. J Pediatr

149(1): 47-52, 2006.

126. Suchi M, MacMullen CM, Thornton PA, Adzick NS, Ganguly A, Ruchelli ED,

Stanley CA: Molecular and immunohistochemical analyses of the focal form of

congenital hyperinsulinism. Mod Path 19: 122-29, 2006.

127. Peranteau WH, Gathalii SM, Pawel B, Hardy O, Alavi A, Stanley CA, Adzick NS:

Multiple ectopic lesions of focal islet adenomatosis identified by positron emission

tomography scan in an infant with congenital hyperinsulinism. J Pediatr Surg

42(1): 188-92, Jan 2007.

128. Hardy OT, Hernandez-Pampaloni M, O'Rourke S, Wintering N, Saffer JR, Suchi M,

Ruchelli E, Zhuang H, Ganguly A, Freifelder R, Adzick NS, Alavi A, Stanley

CA: Diagnosis and localization of focal congenital hyperinsulinism by 18F-

fluorodopa PET scan J Pediatr 150(2): 140-45, Feb 2007.

129. Masia R, Deleon DD, Macmullen C, McKnight H, Stanley CA, Nichols CG: A

mutation in the TMD0-L0 region of SUR1 (L225P) causes permanent neonatal

diabetes mellitus (PNDM). Diabetes Feb 22 2007.


130. Yan FF, Lin YW, Macmullen C, Ganguly A, Stanley CA, Shyng SL: Congenital

hyperinsulinism-associated ABCC8 mutations that cause defective trafficking of

ATP-sensitive potassium channels: identification and rescue. Diabetes Jun 15

2007.

131. Hakonarson H, Grant SF, Bradfield JP, Marchand L, Kim CE, Glessner JT, Grabs R,

Casalunovo T, Taback SP, Frackelton EC, Lawson ML, Robinson LJ, Skraban R,

Lu Y, Chiavacci RM, Stanley CA, Kirsch SE, Rappaport EF, Orange JS, Monos

DS, Devoto M, Qu HQ, Polychronakos C: A genome-wide association study

identifies KIAA0350 as a type 1 diabetes gene. Nature Aug 2 2007.

132. Hardy OT, Hernandez-Pampaloni M, Saffer JR, Scheuermann JS, Ernst LM,

Freifelder R, Zhuang H, MacMullen C, Becker S, Adzick NS, Divgi C, Alavi A,

Stanley CA: Accuracy of [18F]fluorodopa positron emission tomography for

diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol

Metab 92(12): 4706-4711, Dec 2007.

133. De Leon DD, Stanley CA: Permanent Neonatal Diabetes Mellitus. ReneReviews at

GeneTests Page: database online, Feb 2008.

134. Li C, Nissim I, Chen P, Buettger C, Najafi H, Daikhin Y, Nissim I, Collins HW,

Yudkoff M, Stanley CA, Matschinsky FM: Elimination of KATP channels in

mouse islets results in elevated [U-13C] glucose metabolism, glutaminolysis, and

pyruvate cycling but a decreased -aminobutyric acid shunt. J Biol Chem 285(25): 17238-49, June 2008.

135. Lin YW, Bushman JD, Yan FF, Haidar S, MacMullen C, Ganguly A, Stanley CA,

Shyng SL: Destabilization of ATP-sensitive potassium channel activity by novel

KCNJ11 mutations identified in congenital hypersinsulinism. J Biol Chem

283(25): 17238-49, Jun 20 2008.

136. Pinney SE, MacMullen C, Becker S, Lin YW, Hanna C, Thornton P, Ganguly A,

Shyng SL, Stanley CA: Clinical Characteristics and Biochemical Mechanisms of

Congenital Hyperinsulinism Associated with Dominant KATP Channel Mutations.

J Clin Invest 118(8): 2877-86, Aug 2008.

137. De Len DD, Li C, Delson MI, Matschinsky FM, Stanley CA, Stoffers DA:

Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering

cAMP in pancreatic beta-cells and inhibiting insulin secretion. J Biol Chem

283(38): 25786-93, Sept 19 2008.

138. Kelly A, Tang R, Becker S, Stanley CA: Poor specificity of low growth hormone and

cortisol levels during fasting hypoglycemia for the diagnoses of growth hormone

deficiency and adrenal insufficiency. Pediatrics 122(3): e522-2528, Sept 2008.


139. Grant SF, Qu HQ, Bradfield JP, Marchand L, Kim CE, Glessner JT, Grabs R, Taback

SP, Frackelton EC, Eckert AW, Annaiah K, Lawson ML, Otieno FG, Santa E,

Shaner JL, Smith RM, Skraban R, Imielinski M, Chiavacci RM, Grundmeier RW,

Stanley CA, Kirsch SE, Waggott D, Paterson AD; The DCCT/EDIC Research

Group, Monos DS, Polychronakos C, Hakonarson H.: Follow up analysis of

genome-wide association data identifies novel loci for type 1 diabetes. Diabetes

58(1): 290-5, Jan 2009.

140. Pratt EB, Yan FF, Gay JW, Stanley CA, Shyng SL: Sulfonylurea receptor 1 mutations

that cause opposite insulin secretion defects with chemical chaperone exposure. J

Biol Chem 284(12): 7951-9, Mar 2009.

141. Sayed S, Langdon DR, Odili S, Chen P, Buettger C, Schiffman AB, Suchi M, Taub R,

Grimsby J, Matschinsky FM, Stanley CA.: Extremes of clinical and enzymatic

phenotypes in children with hyperinsulinism due to glucokinase activating

mutations. Diabetes 58(6): 1419-27, Jun 2009.

142. Bushman JD, Gay JW, Tewson P, Stanley CA, Shyngs SL: Characterization and

functional restoration of a potassium channel Kir6.2 pore mutation identified in

congenital hyperinsulinism. J Biol Chem 285(9): 6012-23, Feb 2010.

143. Wang K, Baldassano R, Zhang H, Qu HQ, Imielinski M, Kugathasan S, Annese V,

Dubinsky M, Rotter JI, Russell RK, Bradfield JP, Sleiman PMA, Glessner JT,

Walters T, Hou C, Kim C, Frankelton EC, Garris M, Doran J, Romano C, Ctassi C,

Van Limbergen J, Guthery S, Denson L, Piccoli D, Silverberg M, Stanley CA,

Monos D, Wilson DC, Griffiths A, Grant SFA, Satsangi J, Polychronakos C,

Hakonarson H: Comparative genetic analysis of inflammatory bowel disease and

type 1 diabetes implicates multiple loci with opposite effects. Hum Mol Genet Feb

22 2010 Notes: Epub ahead of print.

144. Laje P, Halaby L, Adzick NS, Stanley CA: Necrotizing enterocolitis in neonates

receiving octreotide for the management of congenital hyperinsulinism. Pediatr

Diabetes 11(2): [Epub ahead of print] Mar 2010.

145. Dagli AI, Lee PJ, Correia CE, Rodriguez C, Bhattacharya K, Steinkrauss L, Stanley

CA, Weinstein DA: Pregnancy in glycogen storage disease type Ib: gestational care

and report of first successful deliveries. J Inherit Metab Dis Apr 13 2010 Notes:

[Epub ahead of print]

146. Laje P, Stanley CA, Adzick NS.: Intussusception after pancreatic surgery in children:

a case series. J Pediatr Surg 45(7): 1496-99, July 2010.


147. Li C, Chen P, Palladino A, Narayan S, Russell LK, Sayed S, Xiong G, Chen J, Stokes

D, Butt YM, Jones PM, Collins HW, Cohen NA, Cohen AS, Nissim I, Smith TJ,

Strauss AW, Matschinsky FM, Bennett MJ, Stanley CA: Mechanism of

Hyperinsulinism in Short-chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency

Involves Activation of Glutamate Dehydrogenase. J Biol Chem 285(41): 31806 -

18, Oct 2010.

148. Pingul MM, Hughes N, Wu A, Stanley CA, Gruppuso PA.: Hepatocyte nuclear factor

4 gene mutation associated with familial neonatal hyperinsulinism and maturity-onset diabetes of the young. J Pediatr 158(5): 852-4, May 2011.

149. Macmullen CM, Zhou Q, Snider KE, Tewson PH, Becker SA, Aziz AR, Ganguly A,

Shyng SL, Stanley CA: Diazoxide-unresponsive congenital hyperinsulinism in

children with dominant mutations of the beta-cell sulfonylurea receptor SUR1.

Diabetes 60(6): 1797-804, June 2011.

150. Li C, Li M, Chen P, Narayan, S, Matschinsky FM, Bennett MJ, Stanley CA, Smith TJ:

Green Tea Polyphenols Control Dysregulated Glutamate Dehydrogenase in

Transgenic Mice by Hijacking the ADP Activation Site. J Biol Chem Aug 10 2011.

151. Doliba N, Li C, Qin W, Najafi H, Liu C, Buettger C, Weik-Collins H, Stanley CA,

Wilson DF, Grimsby J, Sarabu R, Naji A, Matschinsky FM: Glucokinase

Activation Repairs Defective Bioenergetics of Islets of Langerhans Isolated from

Type-2 Diabetics. Am J Physiol Endocrinol Metab Sept 27 2011.

152. Li M, Li C, Allen A, Stanley CA, Smith TJ.: The structure and allosteric regulation of

glutamate dehydrogenase. Neurochem Int. 59(4): 445-55, Sept 2011.

153. Doliba NM, Qin W, Najafi H, Liu C, Buettger CW, Sotiris J, Collins HW, Li C,

Stanley CA, Wilson DF, Grimsby J, Sarabu R, Naji A, Matschinsky FM:

Glucokinase activation repairs defective bioenergetics of islets of Langerhans

isolated from type 2 diabetics. Am J Physiol Endocrinol Metab 302(1): E87-E102.

Epub 2011 Sep 27, Jan 1 2012.

154. Laje P, Stanley CA, Palladino AA, Becker SA, Adzick NS.: Pancreatic head resection

and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of

congenital hyperinsulinism. J Pediatr Surg 47(1): 130-5, Jan 2012.

155. Stanley CA, et al: Genetic Disorders of the Beta Cell: a Symposium held at The

Children's Hospital of Philadelphia. Ped Diabetes 13: 344-368, June 2012.

156. Calabria AC, Li C, Gallagher PR, Stanley CA, De Len DD.: GLP-1 Receptor

Antagonist Exendin-(9-39) Elevates Fasting Blood Glucose Levels in Congenital

Hyperinsulinism Owing to Inactivating Mutations in the ATP-Sensitive K+

Channel. Diabetes 61(10): 2585-91, Oct 2012.


157. Stanescu DE, Hughes N, Kaplan B, Stanley CA, De Len DD.: Novel presentations of

congenital hyperinsulinism due to mutations in the MODY genes: HNF1A and

HNF4A. J Clin Endocrinol Metab 97(10): E2026-30, Oct 2012.

158. Narayan SB, Master SR, Sireci AN, Bierl C, Stanley PE, Li C, Stanley CA, Bennett

MJ: Short-Chain 3-Hydroxyacyl-Coenzyme A Dehydrogenase Associates with a

Protein Super-Complex Integrating Multiple Metabolic Pathways. PLoS ONE

7(4): e35048, 2012 Notes: doi:10.1371/hournal.pone.0035048.

159. Palladino Andrew A, Chen Jie, Kallish Staci, Stanley Charles A, Bennett Michael J:

Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry:

acyl-CoA profiles in short-chain fatty acid oxidation defects. Mol Genet Metab

107(4): 679-83, Dec 2012.

160. Laje P, States LJ, Becker SA, Palladino AA, Stanley CA, Adzick NS: Accuracy of

PET/CT Scan in the diagnosis of the focal form of congenital hyperinsulinism. J

Pediatr Surg 48(2): 388-93, Feb 2013.

161. Li C, Liu C, Nissim I, Chen J, Chen P, Doliba N, Zhang T, Nissim I, Daikhin Y,

Stokes D, Yudkoff M, Bennett MJ, Stanley CA, Matschinsky FM, Naji A:

Regulation of Glucagon Secretion in Normal and Diabetic Human Islets by

Gamma-Hydroxybutyrate and Glycine. J Biol Chem 288(6): 3938-51, Feb 2013.

162. Snider K E, Becker S, Boyajian L, Shyng S-L, Macmullen C, Hughes N, Ganapathy

K, Bhatti T, Stanley C A, Ganguly A: Genotype and Phenotype Correlations in 417

Children With Congenital Hyperinsulinism. J Clin Endocrinol Metab 98(2):

E355-63, Feb 2013.

163. Faletra F, Snider K, Shyng SL, Bruno I, Athanasakis E, Gasparini P, Dionisi-Vici,

Ventura A, Zhou Q, Stanley CA, Burlina A: Co-inheritance of two ABCC8

mutations causing an unresponsive congenital hyperinsulinism: Clinical and

functional characterization of two novel ABCC8 mutations. Gene 516(1): 122-5,

Mar 2013.

164. Huang T, Kelly A, Becker SA, Cohen MS, Stanley CA: Hypertrophic

cardiomyopathy in neonates with congenital hyperinsulinism. Arch Dis Child Fetal

Neonatal Ed. 98(4), Jul 2013 Notes: [Epub ahead of print]

165. Laje P, Palladino AA, Bhatti TR, States LJ, Stanley CA, Adzick NS: Pancreatic

surgery in infants with Beckwith-Wiedemann Syndrome and Hyperinsulinism. J

Pediatr Surg 48(12): 2511-16, Dec 2013.

166. Peranteau WH, Palladino AA, Bhatti TR, Becker SA, States LJ, Stanley CA, Adzick

NS: The surgical management of insulinomas in children. J Pediatr Surg 48(12):

2517-24, Dec 2013.


167. Pinney SE, Ganapathy K, Bradfield J, Stokes D, Sasson A, Mackiewicz K,

Boodhansingh K, Hughes N, Becker S, Givler S, Macmullen C, Monos D, Ganguly

A, Hakonarson H, Stanley CA.: Dominant Form of Congenital Hyperinsulinism

Maps to HK1 Region on 10q. Horm Res Paediatr 80(1): 18-27, 2013.

168. Tegtmeyer LC, Rust S, van Scherpenzeel M, Ng BG, Losfeld ME, Timal S, Raymond

K, He P, Ichikawa M, Veltman J, Huijben K, Shin YS, Sharma V, Adamowicz M,

Lammens M, Reunert J, Witten A, Schrapers E, Matthijs G, Jaeken J, Rymen D,

Stojkovic T, Laforet P, Petit F, Develaux I, Piraud M, Stanley CA, Matalon R,

Burda P, Seyyedi S, Debus V, Socha P, Sykut-Cegielska J, van Spronsen F, de

Meirleir L, Vajro P, DeClue T, Ficicioglu C,Wada Y, Wevers RA, Vanderschaeghe

D, Callewaert N, Fingerhut R, van Schaftingen E, Freeze HH, Morava E, Lefeber

DJ, Marquardt T: Multiple phenotypes in phosphoglucomutase 1 deficiency. New

Eng J Med 370(6): 533-42, Feb 2014.

169.

170.

Research Publications, peer-reviewed reviews:

1. Li M, Li C, Allen A, Stanley CA, Smith TJ.: The structure and allosteric regulation of

mammalian glutamate dehydrogenase. Arch Biochem Biophys 519(2): 69-80, Mar

15 2012.

Contributions to peer-reviewed research publications, participation cited but not by

authorship:

[none]

Research Publications, non-peer reviewed:

[none]

Abstracts (Last 3 years):

1. Conlin LK, Thiel BD, Mulchandani S, Kalish JM, Snider KE, Bhatti TR, Ernst LM,

Biegel JA, Spinner NB, Stanley CA, Deardorff MA: Detection and quantification

of mosaic isodisomy in Beckwith-Wiedemann patients using SNP arrays.

"Monogenic Disorders of Insulin Secretion: Congenital Hyperinsulinism and

Neonatal Diabetes" Symposium, The Children's Hospital of Philadelphia March

2012.

(b) (4)

(b) (4)


2. Hughes N, Snider K, Carrie Coleman-Campbell C, Sayed S, Shen G, Boyajian L,

Ganguly A, Stokes D, Stanley CA: Mitochondrial Uncoupling Protein 2 (UCP2)

Mutations in CHOP Hyperinsulinism (HI) Patients. "Monogenic Disorders of

Insulin Secretion: Congenital Hyperinsulinism and Neonatal Diabetes"

Symposium, The Children's Hospital of Philadelphia March 2012.

3. Kalish JM, Snider K, Conlin KL, Bhatti T, Ganguly A, Stanley CA, Deardorff MA: The

Spectrum of Hyperinsulinism in Mosaic Beckwith-Wiedemann Syndrome:

Dissection of 11p15 Paternal Uniparental Isodisomy. "Monogenic Disorders of



4. Li C, Patel P, Zhang T, Chen P, Givler S, Liu C, Nissim I, Naji A, Matschinsky FM, De

Leon DD, Stanley CA: [U-13C]-glucose metabolism in islets from infants with

KATP hyperinsulinism. "Monogenic Disorders of Insulin Secretion: Congenital

Hyperinsulinism and Neonatal Diabetes" Symposium, The Children's Hospital of

Philadelphia March 2012.

5. Lord K, Snider K, MacMullen C, Becker S, Ganguly A, Stanley CA: "Missing"

Mutations: Post-zygotic Mosaicism in Congenital Hyperinsulinism. "Monogenic

Disorders of Insulin Secretion: Congenital Hyperinsulinism and Neonatal

Diabetes" Symposium, The Children's Hospital of Philadelphia March 2012.

6. Patel P, Li C, Givler S, Matschinsky F, Stanley CA, De Leon DD: Functional Evaluation

of Islets from Infants with KATP Hyperinsulinism. "Monogenic Disorders of



7. Pinney SE, Ganapathy K, Bradfield J, Givler S, Becker S, Hughes N, Stokes D, Stanley

CA: Novel form of Autosomal Dominant Hyperinsulinism Maps to Chromosome

10q21. "Monogenic Disorders of Insulin Secretion: Congenital Hyperinsulinism

and Neonatal Diabetes" Symposium, The Children's Hospital of Philadelphia

March 2012.

8. Snider KE, Ganguly A, Becker S, Boyajian L, Shyng SL, MacMullen C, Hughes N,

Ganapathy K, Bhatti T, Lee A, Stanley CA: Genotype-phenotype correlation in 440

children with congenital hyperinsulinism (HI). "Monogenic Disorders of Insulin

Secretion: Congenital Hyperinsulinism and Neonatal Diabetes" Symposium, The

Children's Hospital of Philadelphia March 2012.

9. Stanescu DE, Hughes N, Kaplan B, Stanley CA, De Leon DD: Novel Presentations of

Congenital Hyperinsulinism due to Mutations in the MODY genes: HNF1A and

HNF4A. "Monogenic Disorders of Insulin Secretion: Congenital




10. States LJ, Becker S, Zhuang H, Adzick NS, Stanley CA: Detection of a Focal Lesion of

Congenital Hyperinsulinism using 18[F]-DOPA PET/CT: Lessons learned from

100 consecutive studies with histologic confirmation performed at the Children's

Hospital of Philadelphia. "Monogenic Disorders of Insulin Secretion: Congenital



11. Bennett MB, Narayan SB, Master SR, Sireci A, Bierl C, Stanley PE, Li C, Stanley CA:

SCHAD Associates with a Protein Complex Centered on Glutamate Metabolism.

Poster at International Congress of Inborn Errors of Metabolism, Barcelona, Spain

Sept 2012.

12. Peranteau WH, Palladino AA, Bhatti TR, Stanley CA, Adzick NS: The Surgical

Management of Insulinomas. American Academy of Pediatrics, New Orleans

October 2012.

13. Bennett MB, Narayan SB, Master SR, Beri C, Stanley PE, Li C, Stanley CA: SCHAD

Associates with a Protein Complex Centered on Glutamate Metabolism. Poster at

International Congress of Inform Errors of Metablism, Barcelona, Spain 2012.


PET/CT scan in the diagnosis of the focal form of congenital hyperinsulinism.

British Association of Pediatric Surgeons 2012.


PET/CT scan in the diagnosis of the focal form of congenital hyperinsulinism.

British Association of Pediatric Surgeons 2012.

16. Peranteau WH, Palladino AA, Bhatti TR, Stanley CA, Adzick NS: The Surgical

Management of Insulinomas. American Academy of Pediatrics, New Orleans, LA

2012.

17. Ferrara CT,Stanley CA, Kelly A: Hyperinsulinism Profile: Emerging Biomarkers for

Diagnosing Disease. Poster presentation. Pediatric Academic Society/Pediatric

Endocrine Society Meeting, Washington, DC May 2013.

18. Misfeldt AA, Freeze HH, Morava E, Ficicioglu C, Stanley CA : Hypoglycemia and

Increased Insulin Secretion in a New Form of Glycogen Storage Disease Due to

Phosphoglucomutase-1 Deficiency. Platform presentation. Pediatric Academic

Society/Pediatric Endocrinology Society Meeting. Washington, DC May 2013.

19. Li CH, Patel P, Givler S, Liu CY, Matschinsky F, Naji A, Stanley CA, De Leon DD:

Functional and Metabolic Evaluation of Islets from Infants with KATP

Hyperinsulinism. 2014 Mid-Atlantic Diabetes Research Symposium, Poster

presentation, NIH January 2014.


20. Kalish JM, Boodhansingh K, Conlin LK, Bhatti T, Ganguly A, Bartolomei M,

Palladino A, Adzick NS, Stanley CA, De Leon DD, Deardorff MA: The Spectrum

of Hyperinsulinism in Mosaic Beckwith-Wiedemann Syndrome: Dissection of

11p15 Paternal Uniparental Isodisomy. Research Poster Day, Children's Hospital

of Philadelphia Feb 2014.

21. Bhatti T, Ganapathy K, Huppmann A, Conlin L, Ernst L, MacMullen C, Becker S,

Ganaguly A, Stanley CA, Ruchelli E: Spectrum Of Histopathologic and Molecular

Features Of Pediatric Insulinomas. 2014 Spring Symposium and Kroc Lecture,

Institute for Diabetes, Obesity, and Metabolism, Perlman School of Medicine,

University of Pennsylvania April 2014 Notes: This poster won the Outstanding

poster award for patient-oriented research in the basic science category.

22. Ferrara CT, Boodhansingh KE, Hughes N, Givler S, Ganguly A, Palmieri F, Stanley

CA: Mitochondrial Uncoupling Protein 2 (UCP2) mutations underlying congenital

hyperinsulinism (HI). 2014 Spring Symposium and Kroc Lecture, Institue of

Diabetes, Obesity, and Metabolism, Perlman School of Medicine, University of

Pennsylvania April 2014.

23. Li CH, Misfeldt A, Patel P, Boodhansingh K, Palladino A, Bhatti TR, Adzick NS,

Stanley CA, De Leon DD: KATP channel dysfunction in beta-cell leads to

hypoglycemia in Beckwith-Wiedemann syndrome. American Diabetes Association

74th Annual Meeting for poster presentation, San Francisco June 2014.

24.

Editorials, Reviews, Chapters, including participation in committee reports (print or other

media):

1. Stanley CA, Baker L: Hyperinsulinism in infancy: Diagnosis by demonstration of

abnormal response to fasting hypoglycemia. Pediatrics 57: 702-711, 1976.

2. Baker L, Stanley CA: Hyperinsulinism in infancy: A patho-physiologic approach to

diagnosis and treatment. Rec Prog Pediatr Endocrinol. G. Chiumello (eds.).

Academic Press, New York, Page: 89-100, 1977.

3. Stanley CA, Baker L: Hyperinsulinism in infants and children. Pediatr Clin North Am

44: 363-374, 1977.

4. Baker L, Stanley CA: Diabetes in childhood. Endocrinology Metabolic Basis of

Clinical Practice. DeGroot l, Winegrad AI (eds.). Grune and Stratton, New York,

Page: 1057-1060, 1978.

5. Stanley CA, Baker L: Hypoglycemia. Core Textbook of Pediatrics. Kaye R, Oski F,

Barness L (eds.). J.B. Lippincott Co., Philadelphia, Page: 280-305, 1978.

(b) (4)


6. Stanley CA: Intragastric feeding in glycogen storage disease and other disorders of

fasting. Nutrition in Pediatrics. Walker WA, Watkins JB (eds.). Little, Brown &

Co, Boston, Page: 781-794, 1985.

7. Stanley CA, Coates PM: Inherited defects of fatty acid oxidation which resemble Reye's

syndrome. J Natl Reye's Syndrome Fndn 5: 190-200, 1985.

8. Marx B, Stanley CA: Diabetes. Principles and Practice of Clinical Pediatrics. Schwartz

MW, Ludwig S, Curry TA, Charney EB (eds.). Yearbook Med Publish, Chicago,

Page: 348-354, 1987.

9. Stanley CA: A 3-month old infant with a seizure. Clini-Pearls, Creative Medical

Publications, Inc., New York 4: 1-4, 1987.

10. Stanley CA: New genetic defects in mitochondrial fatty acid oxidation and carnitine

deficiency. Adv Pediatr(34), 59-88, 1987.

11. Treem W, Coates P, Stanley, CA: Genetically determined abnormalities of

mitochondrial fatty acid oxidation. Am Assoc for the Study of Liver Disease,

Annual Course Syllabus 1988.

12. Hale DE, Stanley CA, Coates PM: The long-chain acyl-CoA dehydrogenase

deficiency. Prog Clin Biol Res 321: 303-311, 1990.

13. Hale DE, Stanley CA, Coates PM: Genetic defects of acyl-CoA dehydrogenases:

Studies using an electron transfer flavoprotein reduction assay Prog Clin Biol Res

321: 333-348, 1990.

14. Marx B, Stanley CA: Diabetes Mellitus. Pediatric Primary Care. Schwartz MW,

Charney EB, Curry TA, Ludwig S (eds.). Yearbook Medical Publishers, Chicago,

Page: 423-429, 1990.

15. Stanley CA: Disorders of fatty acid oxidation. Inborn Metabolic Diseases Diagnosis

and Treatment. Fernandes J, Bremer E, Saudubray J-M (eds.). Springer Verlag,

Page: 394-410, 1990.

16. Stanley CA, Hale DE, Coates PM: Medium-chain acyl-CoA dehydrogenase deficiency.

Prog Clin Biol Res(321), 291-302, 1990.

17. Stanley CA, Treem WR, Hale DE, Coates PM: A genetic defect in carnitine transport

causing primary carnitine deficiency. Prog Clin Biol Res 321: 457-464, 1990.

18. Baker L, Thornton PS, Stanley CA: Management of hyperinsulinism in infants J. Peds

(Editorial Commentary) 119: 755-757, 1991.


19. Bennett MJ, Hale DE, Coates PM, Stanley CA: Postmortem recognition of fatty acid

oxidation disorders. Pediatric Pathology 11: 361-366, 1991.

20. Stanley CA: Disorders of carbohydrate metabolism. Pediatric Gastrointestinal Disease.

Walker WA, et al (eds.). BC Decker, Philadelphia, Page: 936-943, 1991.

21. Stanley CA: Neonatal carnitine metabolism. Principles of Neonatal Metabolism.

Cowett RM (eds.). Springer Verlag, Page: 465-471, 1991.

22. Coates PM, Stanley CA: Inherited disorders of mitochondrial fatty acid oxidation. Prog

Liver Dis 10: 123-38, 1992.

23. Schmidt-Sommerfeld E, Penn D, Duran M, Rinaldo P, Bennett MJ, Santer R, Stanley

CA: Detection and quantitation of acylcarnitines in plasma and blood spots from

patients with inborn errors of fatty acid oxidation. Prog Clin Biol Res 375: 355-362,

1992.

24. Stanley CA: Plasma and mitochondrial membrane carnitine transport defects. Prog

Clin Biol Res 375: 289-300, 1992.

25. Stanley CA: Disorders of fatty acid oxidation Nelson Textbook of Pediatrics W.B.

Sauders, Philadelphia, 14th edition, Page: 336-338, 1992.

26. Thornton PS, Berry GT, Stanley CA: Disorders of intermediary metabolism: Organ

system failure. Pediatric Critical Care. Holbrook P (eds.). Page: 725-740, 1992.

27. Stanley CA: Familial hyperinsulinism: Successful conservative management. 1993

Yearbook of Pediatrics(Editorial Commentary) 1993.

28. Stanley CA, Hale DE: Genetic disorders of mitochondrial fatty acid oxidation. Curr

Opin Pediatr 6(4): 476-81, Aug 1994.

29. Stanley CA: Disorders of fatty acid oxidation. Inborn Metabolic Diseases: Diagnosis

and Treatment, 2nd Edition. Fernandes J, Saudubray JM, Van Den Berghe T

(eds.). Springer-Verlag, Berlin, Heidenberg, New York, Page: 133-143, 1994.

30. Stanley CA: Defects in metabolism of lipids: Disorders of Mitochondrial Fatty Acid

Oxidation. Nelson Textbook of Pediatrics, 15th Edition. Behrman RE, Kliegman

RM, Arvin AM, Nelson WE (eds.). WB Saunders, Philadelphia, Page: 360-363,

1994.

31. Stanley CA: Carnitine disorders. Adv Pediatr 42: 209-42, 1995.

32. Stanley CA: Medium-chain acyl-CoA dehydrogenase deficiency. Neurobase. Arbor

Publishing Company, 1995.


33. Stanley CA: Carnitine/acylcarnitine translocase deficiency. Neurobase. Arbor


34. Stanley CA: Carnitine palmitoyl transferase-1 (CPT-1) deficiency. Neurobase. Arbor


35. Katz L, Stanley CA: Disorders of Glucose and Other Sugars. Intensive Care of the

Fetus and Neonate. Philadelphia, Mosby - Yearbook, Page: 982-992, 1996.

36. Stanley CA: Hyperinsulinism in infants and children. Pediatr Clin North Am 44(2):

363-74, Apr 1997.

37. Baker L, Stanley CA: Neonatal hypoglycemia. Curr Ther Endocrinol Metab 6: 409-13,

1997.

38. Satin-Smith MS, Stanley CA: Diabetes mellitus. Pediatric Primary Care. Schwartz

MW, Charney EB, Curry TA, Ludwig S (eds.). Yearbook Medical Publishers,

Chicago, Page: 523-529, 1997.

39. Stanley CA, Baker L: Definition of hypoglycemia in the neonatal period. Curr Ther

Endocrinol Metab 6: 409-413, 1997.

40. Nestorowicz A, Stanley CA, Landau H, Glaser B, Permutt MA: Familial

hyperinsulinism in Ashkenazi Jews. Advances in Jewish Genetic Diseases.

Desnick RJ (eds.). 1998.

41. Permutt MA, Chui K, Ferrer J, Glaser B, Inoue H, Nestorowicz A, Stanley C, Tanizawa

Y: Genetics of Type II Diabetes. Recent Prog Horm Res 53: 201-215, 1998.

42. Stanley CA: Dissecting the spectrum of fatty acid oxidation disorders. J Pediatr

(editorial) 132: 384-386, 1998.

43. Stanley CA: Defects in Metabolism of Lipids. Endocrinology, Chapter 72, 90, 94, 96,

98 Pediatrics at a Glance, 1998.

44. Stanley CA: Defects in Metabolism of Lipids. Nelson Textbook of Pediatrics 1998.

45. Stanley CA: Neonatal carnitine metabolism. Principles of Neonatal Metabolism, 2nd

Edition. Cowett RM (eds.). Springer Verlaq, 1998.

46. Satin-Smith MS, Stanley CA: Hypoglycemia. Gellis and Kagan's Current Pediatric

Therapy. W.B. Saunders, 16: 772-774, 1999.


47. Stanley CA: The hyperinsulinism-hyperammonemia syndrome: gain-of-function

mutations of glutamate dehydrogenase. Genetic Insights in Pediatric

Endocrinology and Metabolism. O'Rahilly S, Dunger DB (eds.). Bristol:

BioScientifica, Ltd, Page: 23-30, 1999.

48. Stanley CA, Baker L: The causes of neonatal hypoglycemia N Engl J Med 340:

1200-1201, 1999.

49. Ferry RJ, Kelly A, Stanley CA: The hyperinsulinism/hyperammonemia syndrome.

Home page of The Japanese Pediatric Endocrinology Society (Tokyo, Japan

(www.pnu.cojp/gh) 86: 1782-87, 2000.

50. Stanley CA: Disorders of carbohydrate metabolism. Pediatric Gastrointestinal Disease.

Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB (eds.).

Philadelphia: Decker, Chapter 60, 2000.

51. Stanley CA: Lethargy and vomiting in a 14 month-old boy. Pediatrics: A

Problem-Based Review. Burg FD, Vaughan VC, Nelson KG (eds.). W.B. Saunders

Company, Philadelphia, Page: 232-235, 2000.

52. Stanley CA: Hypoglycemia. Essence of Office Pediatrics. Stockman JA, Lohr JA

(eds.). WB Saunders, Page: 213, 2000.

53. Stanley CA: Disorders of fatty acid oxidation. Inborn Metabolic Diseases (Third

Edition). Fernandes J, Saudubray JM, van den Berghe (eds.). Heidelberg:

Springer-Verlag, Page: 139-150, 2000.

54. Stanley CA, Alter C, Thornton PS, Katz LL, Weinzimer S, Kelly A, et al:

Hypoglycemia. YourDoctor.Com. Stanley CA, section editor, Rosenfeld R, editor,

Robert Parry, M.D. Executive Editor (eds.). 2000.

55. Stanley CA, Thornton PS: Leucine-sensitive hypoglycemia unraveled. Topical

Endocrinology. ; Chapterhouse Codex, Ltd Page: 9-12, December 2000.

56. Kelly A, Stanley CA: Disorders of glutamate metabolism. Ment Retard Dev Disabil

Res Rev 7(4): 287-95, 2001.

57. Ng DD, Ferry RJ, Jr., Kelly A, Weinzimer SA, Stanley CA, Katz LE. : Acarbose

treatment of postprandial hypoglycemia in children after Nissen fundoplication. J

Pediatr 139(6): 877-79, 2001.

58. Stanley CA: Advances in diagnosis and treatment of hyperinsulinism in infants and

children. J Clin Endocrinol Metab 87(11): 4857-59, 2002.


59. Stanley, CA, Thornton, PS, Finegold, DN, Sperling, MA: Hypoglycemia in Neonates

and Infants. 2nd Edition of Pediatric Endocrinology. Mark A. Sperling, M.D (eds.).

WB Saunders, 7: 135-159, 2002.

60. Thornton PA, Finegold DN, Stanley CA: Hypoglycemia in the Infant and Child. 2nd

Edition of Pediatric Endocrinology. Mark A. Sperling, M.D. (eds.). WB Saunders,

13: 367-384, 2002.

61. Stanley CA: Hypoglycemia. Pediatric Endocrinology: A Practical Clinical Guide. S.

Radovick & M.H. MacGillivray (eds.). Humana Press Inc, Page: 511-521, 2003.

62. Stanley CA, Caplin N: Pathophysiology of Hypoglycemia. 3rd Edition of Fetal and

Neonatal Physiology. Polin, Fox, & Abman (eds.). WB Saunders 51: 494-499,

2003.

63. Stanley CA: Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory

role of glutamate dehydrogenase in ammonia metabolism. Mol Genet Metab(81

Suppl 1), S45-51, Apr 2004.

64. Hussain K, Aynsley-Green A, Stanley CA: Medications used in the treatment of

hypoglycemia due to congenital hyperinsulinism of infancy (HI). Pediatr

Endocriniol Rev(2 Suppl 1), 163-7, Nov 2004.

65. Stanley CA: Carnitine deficiency disorders in children. Ann N Y Acad Sci 1033: 42-51,

November 2004.

66. Fourtner SH, Stanley CA: Genetic and Nongenetic Forms of Hyperinsulinism in

Neonates. NeoReviews 5(9): 370-76, 2004.

67. Kelly A, Changhong L, Stanley CA: Glucokinase/glutamate dehydrogenase

interactions in the GDH form of congenital hyperinsulinism. Glucokinase and

Glycemic Disease: From Basics to Novel Therapeutics. Front Diabetes.

Matschinsky FM & Magnuson MA (eds.). Basel Karger, Page: 110-24, 2004.

68. Stanley CA: Carnitine Deficiency Disorders in Children. Annals of the New York

Academy of Sciences 1033: 42-51, 2004.

69. Stanley CA: Hypoglycemia. 2nd Edition of Practical Strategies in Pediatric Diagnosis

and Therapy Greenbaum, Lye (eds.). Kliegman, 61: 1121-31, 2004.

70. Stanley CA, Pallotto EK: Disorders of Carbohydrate Metabolism. 8th Edition of

Avery's Diseases of the Newborn. H. William Taeusch, M.D., Roberta A. Ballard,

M.D., Christine A. Gleason, M.D. (eds.). WB Saunders, 93: 1410-22, 2004.


71. Katz LL, Stanley CA: Disorders of Glucose and Other Sugars. Intensive Care of The

Fetus & Neonate, 2nd Edition Spitzer MD, Alan (eds.). Elsevier, Inc, Page:

1167-78, Apr 2005.

72. DeLeon DD, Stanley CA: When to Screen for Neonatal Hypoglycemia. When to

Screen in Obstetrics and Gynecology, 2nd Edition. Wildschut, Weiner, Peters

(eds.). Saunders Elsevier, 40: 483-490, 2006.

73. Hardy OT, Stanley CA: Hyperinsulinism of Infancey: Localization of Focal Forms.

Practical Pediatric PET Imaging. Martin Charron, M.D., FRCP (eds.). Springer,

26B: 479-484, 2006.

74. Stanley CA: Parsing ketotic hypoglycaemia. Archieves of Disease in Childhood 6,

2006.

75. Stanley CA: Hypoglycemia in the neonate. Pediatr Endocrinol Rev(4 Suppl 1), 76-81,

Dec 2006.

76. Stanley Ca, Bennett MJ, Mayatepek E: Disorders of Mitochondrial Fatty Acid

Oxidation and Related Metabolic Pathways. Inborn Metabolic Diseases: Diagnosis

and Treatment. John Fernandes, Jean-Marie Saudubray, Georges van den Berghe

and John H. Walter (eds.). Springer Medizin Verlag Heidelberg, Germany, 4th

edition: 175-190, 2006.

77. De Leon D, Stanley CA: Mechanisms of Disease: advances in diagnosis and treatment

of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metabol(3), 57-68, Jan

2007.

78. Stanley CA, Bennett MJ: Disorders of Mitochondiral Fatty Acid Oxidation. Nelson

Textbook of Pediatrics. Kliegman, Behrman, Jenson, Stanton (eds.). Saunders

Elsevier, 18th Edition: 567-72, 2007.

79. Palladino AA, Bennett MJ, Stanley CA: Hyperinsulinism in infancy and childhood:

when an insulin level is not always enough. Clin Chem 54(2): 256-63, Feb 2008.

80. Smith TJ, Stanley CA: Untangling the glutamate dehydrogenase allosteric nightmare.

Trend Biochem Sci 33(11): 557-64, Nov 2008.

81. Kelly A, Stanley CA: Neurological aspects in hyperinsulinism-hyperammonaemia

syndrome. Developmental Medicine & Child Neurology 50(12): 888, Dec 2008.

82. Kelly A, Stanley CA: Hyperinsulinism. Pediatric Endocrinology and Inborn Errors of

Metabolism. Sarafoglou K (eds.). McGraw Hill, Page: 39-50, 2008.


83. Palladino AA, Bennett MJ, Stanley CA: Hyperinsulinism in infancy and childhood:

when an insulin level is not always enough. Ann Bio Clin 67(3): 245-54, May-Jun

2009.

84. Stanley CA: Regulation of glutamate metabolism and insulin secretion by glutamate

dehydrogenase in hypoglycemic children. Am J Clin Nutr 90(3): 862S-866S, Sept

2009.

85. Bennett M, Stanley CA: Carnitine palmitoyl transferase II deficiency. orphanet

ORPHA157, April 2010.

86. Bennett M, Stanley CA: Carnitine palmitoyl transferase II deficiency, neonatal form.

orphanet ORPHA228308, May 2010.

87. Bennett M, Stanley CA: Carnitine palmitoyl transferase II deficiency myopathic form.

orphanet ORPHA228302, May 2010.

88. Bennett M, Stanley CA: Carnitine palmitoyl transferase II deficiency, severe infantile

form. orphanet ORPHA228305, May 2010.

89. Thornton PS, Stanley CA, De Len D, Kelly A, Sperling MA, Wolfsdorf JI, Haymond

M: Research initiatives in neonatal hypoglycemia. J Pediatr 156(5): 862-3, May

2010.

90. Palladino AA, Stanley CA: The hyperinsulinism/hyperammonemia syndrome. Rev

Endocr Metab Disord 11(3): 171-178, Sep 2010.

91. Marquard J, Palladino AA, Stanley CA, Mayatepek E, Meissner T.: Rare forms of

congenital hyperinsulinism. Semin Pediatr Surg 2011 20(1): 38-44, Feb 2011.

92. Laje P, Halaby L, Adzick NS, Stanley CA: Necrotizing enterocolitis in neonates

receiving octreotide for the management of congenital hyperinsulinism. Pediatr

Diabetes. 2010 Mar;11(2):142-7. 11(2): 142-7, Mar 2011.

93. Palladino AA, Stanley CA: Nesidioblastosis No Longer! It's All About Genetics. J Clin

Endocrinol Metab 96(3): 617-9, Mar 2011.

94. Stanley CA: Two genetic forms of hyperinsulinemic hypoglycemia caused by

dysregulation of glutamate dehydrogenase. Neurochem Int 59(4): 465-72, Sep

2011.

95. Stanley CA: Two genetic forms of hyperinsulinemic hypoglycemia caused by

dysregulation of glutamate dehydrogenase. Neurochem Int 59(4): 465-72, Sept

2011.


96. Bennett MJ, Stanley CA: Plasma Membrane Carnitine Transporter Defect. The Online

Metabolic & Nolecular Sases of Inherited Disease. Valle D, Vogelstein B, Kinzler

KW, Antonarakis SE, Ballagio A, Gibson KM, Mitchell G (eds.).

McGraw-Hill,(101.1), 2011.

97. De Leon DD, Stanley CA: Permanent Neonatal Diabetes Mellitus. GeneReview. Pagan

RA, Adam MP, Bird TD, Dolan CR, Fong CT, Smith RJH, Stephen K (eds.).

GeneReviews [Internet] 2011.

98. DeLeon D, Stanley CA: Hypoglycemia. Pediatric Endocrinology: A Practical Clinical

Guide. Sally Radovick, MD and Margaret H. MacGillivray, MD (eds.). Springer,

2nd edition, 2011.

99. Hardy OT, Stanley CA: Hypoglycemia. Fetal and Neonatal Physiology: Expert Consult

- Online and Print, 2-Volume Set Richard A. Polin, William W. Fox, M.D.,

Steven H. Abman (eds.). Elsevier - Health Sciences Division Page: 568-575,

2011.

100. Stanley CA, Bennett MJ: Disorders of Mitochondrial Fatty Acid Beta-Oxidation.

Nelson Textbook of Pediatrics, 19th Edition. Robert M. Kliegman, Bonita F.

Stanton, Joseph W. St. Geme III, Nina F. Schor, Richard E. Behrman (eds.).

Elsevier Saunders, Page: 456-462, 2011.

101. De Leon DD, Stanley CA: Pathophysiology of Diffuse ATP-Sensitive Potassium

Channel Hyperinsulinism. Frontiers in Diabetes: Monogenic hyperinsulinemic

hypoglycemia disorders. Karger, 21, Feb 2012.

102. Kelly A, Palladino A, Stanley CA: Congenital Hyperinsulinism Due to Activating

Mutations of Glutamate Dehydrogenase: The Hyperinsulinism/Hyperammonemia

Syndrome. Frontiers in Diabetes: Monogenic hyperinsulinemic hypoglycemia

disorders. Karger, 21, Feb 2012.

103. Li C, Matschinsky FM, Stanley CA: Amino Acid-Stimulated Insulin Secretion: The

Role of the Glutamine-Glutamate-Alpha-Ketoglutarate Axis. Frontiers in Diabetes:

Monogenic hyperinsulinemic hypoglycemia disorders. Karger, 21, Feb 2012.

104. Sayed S, Matschinsky FM, Stanley CA: Hyperinsulinism Due to Activating

Mutations of Glucokinase. Frontiers in Diabetes: Monogenic hyperinsulinemic

hypoglycemia disorders. Karger, 21, Feb 2012.

105. Stanley CA, Matschinsky FM: Historical Perspective on the Genetic Forms. Frontiers

in Diabetes: Monogenic hyperinsulinemic hypoglycemia diosrders. Karger, 21,

Feb 2012.


106. Li M, Li C, Allen A, Stanley CA, Smith TJ: The structure and allosteric regulation of

mammalian glutamate dehydrogenase. Arch Biochem Biophys 15(2): 69-80, Mar

2012.

107. Blomberg BA, Moghbel MC, Saboury B, Stanley CA, Alavi A.: The value of

radiologic interventions and (18)F-DOPA PET in diagnosing and localizing focal

congenital hyperinsulinism: systematic review and meta-analysis. Mol Imaging

Biol 15(1): 97-105, Feb 2013.

108. De Leon DD, Stanley CA: Determination of insulin for the diagnosis of

hyperinsulinemic hypoglycemia. Best Practice & Research Clinical Endocrinology

& Metabolism 27(6): 763-769, Dec 2013.

109. De Leon DD, Thornton PS, Stanley CA, Sperling MA: Hypoglycemia in Neonates

and Infants. Pediatric Endocrinology, 4th Edition. Mark A. Sperling (eds.).

Saunders, 2013.

110. Diva D. De LeonCharles A. Stanley: Hypoglycemia. Contempory Endocrinology.

Sally Radovick (eds.). Springer, 2013.

111. Kelly A, Stanley CA: Hyperinsulinism. Pediatric Endocrinology and Inborn Errors of

Metabolism, 2nd Edition. Sarafoglou K, Roth KS, Hoffmann GF (eds.). 2013.

112. Langdon DR, Stanley CA, Sperling MA.: Hypoglycemia in the Infant and Child.

Pediatric Endocrinology, 4th Edition. Mark A. Sperling (eds.). Saunders, 2013.

113. Stanley CA: Congenital Hyperinsulinism. Clinical Decision Support: Pediatrics.

Bonney, McMillan, Barrett, Jones (eds.). Decision Support in Medicine, LLC,

2013 Notes: online publication.

114. De Len DD, and Stanley CA: Permanent Neonatal Diabetes Mellitus. GeneReview.

Pagon RA, Adam MP, Bird TD, Dolan CR, Fong CT, Smith RJH, Stephens K

(eds.). GeneReviews [Internet]. Updated, Jan 23 2014.

115. Bennett MJ, Stanley CA, Longo N: Plasma Membrane Carnitine Transporter Defect.

Online Metabolic & Molecular Basis of Inherited Disease. David Valle, MD,

Editor-in-Chief, Arthur L. Beaudet, MD,Bert Vogelstein, MD, Kenneth W.

Kinzler, Ph.D., Stylianos E. Antonarakis, MD, D.Sc., Andrea Ballabio, MD, K.

Michael Gibson, Ph.D., FACMG, Grant Mitchell, MD (eds.). McGraw Hill, March

2014.

116.

(b) (4)


Books:

1. Charles A. Stanley and Diva D. De Leon (Eds): Monogenic Hyperinsulinemic

Hypoglycemia Disorders. Frontiers in Diabetes. Karger, 21, Feb 2012.

Alternative Media:

[none]

Patents:

Smith, Li, Stanley: Methods and Compositions for Modulating Glutamate Dehydrogenase. US

Patent Number 11/263,797, 2005.

DeLeon, Stoffers, Stanley: Methods and Compositions for Treating Hypoglycemic Disorders.

US Patent Number 12/522,274, 2009.

Documents

Stanley Charles CV 1 2015_Redactedh